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Items: 1 to 50 of 122

1.

The Estrogen-Induced miR-19 Downregulates Secretory Leucoprotease Inhibitor Expression in Monocytes.

McKiernan PJ, Smith SGJ, Durham AL, Adcock IM, McElvaney NG, Greene CM.

J Innate Immun. 2019 Jul 2:1-13. doi: 10.1159/000500419. [Epub ahead of print]

2.

A novel mutation in VPS33B gene causing a milder ARC syndrome phenotype with prolonged survival.

Del Brío Castillo R, Squires JE, McKiernan PJ.

JIMD Rep. 2019 Mar 22;47(1):4-8. doi: 10.1002/jmd2.12027. eCollection 2019 May.

3.

Evolving Trends in Liver Transplant for Metabolic Liver Disease in the United States.

McKiernan PJ, Ganoza A, Squires JE, Squires RH, Vockley J, Mazariegos G, Soltys K, Sun Q, Sindhi R.

Liver Transpl. 2019 Jun;25(6):911-921. doi: 10.1002/lt.25433. Epub 2019 Apr 15.

PMID:
30753750
4.

Knockdown of Gene Expression in Macrophages by microRNA Mimic-Containing Poly (Lactic-co-glycolic Acid) Microparticles.

McKiernan PJ, Lynch P, Ramsey JM, Cryan SA, Greene CM.

Medicines (Basel). 2018 Dec 15;5(4). pii: E133. doi: 10.3390/medicines5040133.

5.

Hepatopulmonary Syndrome in Children: A 20-Year Review of Presenting Symptoms, Clinical Progression, and Transplant Outcome.

Warner S, McKiernan PJ, Hartley J, Ong E, van Mourik ID, Gupte G, Abdel-Hady M, Muiesan P, Perera T, Mirza D, Sharif K, Kelly DA, Beath SV.

Liver Transpl. 2018 Sep;24(9):1271-1279. doi: 10.1002/lt.25296.

PMID:
30066494
6.

Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children.

Johansen LC, McKiernan PJ, Sharif K, McGuirk SP.

J Pediatr Gastroenterol Nutr. 2018 Aug;67(2):173-179. doi: 10.1097/MPG.0000000000002006.

PMID:
29668568
7.

Survival of children after liver transplantation for hepatocellular carcinoma.

Baumann U, Adam R, Duvoux C, Mikolajczyk R, Karam V, D'Antiga L, Chardot C, Coker A, Colledan M, Ericzon BG, Line PD, Hadzic N, Isoniemi H, Klempnauer JL, Reding R, McKiernan PJ, McLin V, Paul A, Salizzoni M, Furtado ESB, Schneeberger S, Karch A; European Liver and Intestine Transplant Association.

Liver Transpl. 2018 Feb;24(2):246-255. doi: 10.1002/lt.24994.

8.

Daily variation of NTBC and its relation to succinylacetone in tyrosinemia type 1 patients comparing a single dose to two doses a day.

Kienstra NS, van Reemst HE, van Ginkel WG, Daly A, van Dam E, MacDonald A, Burgerhof JGM, de Blaauw P, McKiernan PJ, Heiner-Fokkema MR, van Spronsen FJ.

J Inherit Metab Dis. 2018 Mar;41(2):181-186. doi: 10.1007/s10545-017-0112-9. Epub 2017 Nov 23.

9.

Portal hypertension and its management in children.

Grammatikopoulos T, McKiernan PJ, Dhawan A.

Arch Dis Child. 2018 Feb;103(2):186-191. doi: 10.1136/archdischild-2015-310022. Epub 2017 Aug 16. Review.

PMID:
28814423
10.

X Chromosome-encoded MicroRNAs Are Functionally Increased in Cystic Fibrosis Monocytes.

McKiernan PJ, Molloy KP, Cryan SA, McElvaney NG, Greene CM.

Am J Respir Crit Care Med. 2018 Mar 1;197(5):668-670. doi: 10.1164/rccm.201707-1417LE. No abstract available.

PMID:
28796536
11.

Attempt to Determine the Prevalence of Two Inborn Errors of Primary Bile Acid Synthesis: Results of a European Survey.

Jahnel J, Zöhrer E, Fischler B, D'Antiga L, Debray D, Dezsofi A, Haas D, Hadzic N, Jacquemin E, Lamireau T, Maggiore G, McKiernan PJ, Calvo PL, Verkade HJ, Hierro L, McLin V, Baumann U, Gonzales E.

J Pediatr Gastroenterol Nutr. 2017 Jun;64(6):864-868. doi: 10.1097/MPG.0000000000001546.

PMID:
28267072
12.

UK guideline on transition of adolescent and young persons with chronic digestive diseases from paediatric to adult care.

Brooks AJ, Smith PJ, Cohen R, Collins P, Douds A, Forbes V, Gaya DR, Johnston BT, McKiernan PJ, Murray CD, Sebastian S, Smith M, Whitley L, Williams L, Russell RK, McCartney SA, Lindsay JO.

Gut. 2017 Jun;66(6):988-1000. doi: 10.1136/gutjnl-2016-313000. Epub 2017 Feb 21. Review.

13.

What Is the Best Blood Sampling Time for Metabolic Control of Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients?

van Dam E, Daly A, Venema-Liefaard G, van Rijn M, Derks TGJ, McKiernan PJ, Rebecca Heiner-Fokkema M, MacDonald A, van Spronsen FJ.

JIMD Rep. 2017;36:49-57. doi: 10.1007/8904_2016_37. Epub 2017 Jan 25.

14.

Expression of X-linked Toll-like receptor 4 signaling genes in female vs. male neonates.

O'Driscoll DN, De Santi C, McKiernan PJ, McEneaney V, Molloy EJ, Greene CM.

Pediatr Res. 2017 May;81(5):831-837. doi: 10.1038/pr.2017.2. Epub 2017 Jan 6.

PMID:
28060792
15.

MiRNA Expression in Cystic Fibrosis Bronchial Epithelial Cells.

Oglesby IK, McKiernan PJ.

Methods Mol Biol. 2017;1509:57-69.

PMID:
27826918
16.

Successful long-term outcome of liver transplantation in late-onset lysosomal acid lipase deficiency.

Sreekantam S, Nicklaus-Wollenteit I, Orr J, Sharif K, Vijay S, McKiernan PJ, Santra S.

Pediatr Transplant. 2016 Sep;20(6):851-4. doi: 10.1111/petr.12748. Epub 2016 Jul 9.

PMID:
27392817
17.

Neurocognitive outcome in tyrosinemia type 1 patients compared to healthy controls.

van Ginkel WG, Jahja R, Huijbregts SC, Daly A, MacDonald A, De Laet C, Cassiman D, Eyskens F, Körver-Keularts IM, Goyens PJ, McKiernan PJ, van Spronsen FJ.

Orphanet J Rare Dis. 2016 Jun 29;11(1):87. doi: 10.1186/s13023-016-0472-5.

18.

Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome.

Sentner CP, Hoogeveen IJ, Weinstein DA, Santer R, Murphy E, McKiernan PJ, Steuerwald U, Beauchamp NJ, Taybert J, Laforêt P, Petit FM, Hubert A, Labrune P, Smit GPA, Derks TGJ.

J Inherit Metab Dis. 2016 Sep;39(5):697-704. doi: 10.1007/s10545-016-9932-2. Epub 2016 Apr 22.

19.

Combined liver and kidney transplantation in children: analysis of renal graft outcome.

Ranawaka R, Lloyd C, McKiernan PJ, Hulton SA, Sharif K, Milford DV.

Pediatr Nephrol. 2016 Sep;31(9):1539-43. doi: 10.1007/s00467-016-3396-3. Epub 2016 Apr 22.

PMID:
27105881
20.

High-throughput profiling for discovery of non-coding RNA biomarkers of lung disease.

McKiernan PJ, Greene CM.

Expert Rev Mol Diagn. 2016;16(2):173-85. doi: 10.1586/14737159.2016.1122526. Epub 2015 Dec 10. Review.

PMID:
26581119
21.

Recurrent acute liver failure due to NBAS deficiency: phenotypic spectrum, disease mechanisms, and therapeutic concepts.

Staufner C, Haack TB, Köpke MG, Straub BK, Kölker S, Thiel C, Freisinger P, Baric I, McKiernan PJ, Dikow N, Harting I, Beisse F, Burgard P, Kotzaeridou U, Lenz D, Kühr J, Himbert U, Taylor RW, Distelmaier F, Vockley J, Ghaloul-Gonzalez L, Ozolek JA, Zschocke J, Kuster A, Dick A, Das AM, Wieland T, Terrile C, Strom TM, Meitinger T, Prokisch H, Hoffmann GF.

J Inherit Metab Dis. 2016 Jan;39(1):3-16. doi: 10.1007/s10545-015-9896-7. Epub 2015 Nov 5.

PMID:
26541327
22.

MicroRNA Dysregulation in Cystic Fibrosis.

McKiernan PJ, Greene CM.

Mediators Inflamm. 2015;2015:529642. doi: 10.1155/2015/529642. Epub 2015 Jun 22. Review.

23.

Biallelic Mutations in NBAS Cause Recurrent Acute Liver Failure with Onset in Infancy.

Haack TB, Staufner C, Köpke MG, Straub BK, Kölker S, Thiel C, Freisinger P, Baric I, McKiernan PJ, Dikow N, Harting I, Beisse F, Burgard P, Kotzaeridou U, Kühr J, Himbert U, Taylor RW, Distelmaier F, Vockley J, Ghaloul-Gonzalez L, Zschocke J, Kremer LS, Graf E, Schwarzmayr T, Bader DM, Gagneur J, Wieland T, Terrile C, Strom TM, Meitinger T, Hoffmann GF, Prokisch H.

Am J Hum Genet. 2015 Jul 2;97(1):163-9. doi: 10.1016/j.ajhg.2015.05.009. Epub 2015 Jun 11.

24.

Interobserver Agreement on Endoscopic Classification of Oesophageal Varices in Children.

D'Antiga L, Betalli P, De Angelis P, Davenport M, Di Giorgio A, McKiernan PJ, McLin V, Ravelli P, Durmaz O, Talbotec C, Sturm E, Woynarowski M, Burroughs AK.

J Pediatr Gastroenterol Nutr. 2015 Aug;61(2):176-81. doi: 10.1097/MPG.0000000000000822.

PMID:
25883057
25.

Outcome of children with hereditary tyrosinaemia following newborn screening.

McKiernan PJ, Preece MA, Chakrapani A.

Arch Dis Child. 2015 Aug;100(8):738-41. doi: 10.1136/archdischild-2014-306886. Epub 2015 Jan 6.

PMID:
25564536
26.

Non-coding RNA as lung disease biomarkers.

Vencken SF, Greene CM, McKiernan PJ.

Thorax. 2015 May;70(5):501-3. doi: 10.1136/thoraxjnl-2014-206193. Epub 2014 Dec 30. Review.

PMID:
25550385
27.

Selenium homocholic acid taurocholate scanning, selenium-75-labeled bile acid, a novel method for testing the function of the terminal ileum in small bowel transplant recipients: a pilot study.

Beath SV, Lanchbury E, Alton H, Mahandru R, Toy M, van Mouirk ID, McKiernan PJ, Hartley J, Kelly DA, Sharif K, Gupte G.

Transplant Proc. 2014 Jul-Aug;46(6):2119-21. doi: 10.1016/j.transproceed.2014.06.036.

PMID:
25131120
28.

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium.

McKiernan PJ, Molloy K, Cryan SA, McElvaney NG, Greene CM.

Int J Biochem Cell Biol. 2014 Jul;52:184-91. doi: 10.1016/j.biocel.2014.02.022. Epub 2014 Mar 12.

PMID:
24631641
29.

Early nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and improves post-transplant renal function.

Bartlett DC, Lloyd C, McKiernan PJ, Newsome PN.

J Inherit Metab Dis. 2014 Sep;37(5):745-52. doi: 10.1007/s10545-014-9683-x. Epub 2014 Feb 11.

PMID:
24515874
30.

Rejection is less common in children undergoing liver transplantation for hepatoblastoma.

Ruth ND, Kelly D, Sharif K, Morland B, Lloyd C, McKiernan PJ.

Pediatr Transplant. 2014 Feb;18(1):52-7. doi: 10.1111/petr.12194. Epub 2013 Dec 16.

PMID:
24341552
31.

Targeting miRNA-based medicines to cystic fibrosis airway epithelial cells using nanotechnology.

McKiernan PJ, Cunningham O, Greene CM, Cryan SA.

Int J Nanomedicine. 2013;8:3907-15. doi: 10.2147/IJN.S47551. Epub 2013 Oct 11.

32.

A comparison of two validated scores for estimating risk of mortality of children with intestinal failure associated liver disease and those with liver disease awaiting transplantation.

Beath SV, Davies P, Mukherjee A, Lloyd C, Sharif K, McKiernan PJ, van Mourik ID, Kelly DA.

Clin Res Hepatol Gastroenterol. 2014 Feb;38(1):32-9. doi: 10.1016/j.clinre.2013.06.001. Epub 2013 Jul 12.

PMID:
23856636
33.

Population pharmacokinetic and pharmacogenetic analysis of tacrolimus in paediatric liver transplant patients.

Jalil MH, Hawwa AF, McKiernan PJ, Shields MD, McElnay JC.

Br J Clin Pharmacol. 2014 Jan;77(1):130-40. doi: 10.1111/bcp.12174.

34.

Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis.

Rawat D, Kelly DA, Milford DV, Sharif K, Lloyd C, McKiernan PJ.

J Pediatr Gastroenterol Nutr. 2013 Aug;57(2):161-6. doi: 10.1097/MPG.0b013e318291e72b.

PMID:
23518487
35.

Changing strategies for organ transplantation in atypical haemolytic uraemic syndrome: a tertiary case series.

Forbes TA, Bradbury MG, Goodship TH, McKiernan PJ, Milford DV.

Pediatr Transplant. 2013 May;17(3):E93-9. doi: 10.1111/petr.12066. Epub 2013 Mar 6.

PMID:
23461281
36.

Plasma succinylacetone is persistently raised after liver transplantation in tyrosinaemia type 1.

Bartlett DC, Preece MA, Holme E, Lloyd C, Newsome PN, McKiernan PJ.

J Inherit Metab Dis. 2013 Jan;36(1):15-20. doi: 10.1007/s10545-012-9482-1. Epub 2012 Mar 29.

PMID:
22456946
37.

Therapeutic modulation of miRNA for the treatment of proinflammatory lung diseases.

Hassan T, McKiernan PJ, McElvaney NG, Cryan SA, Greene CM.

Expert Rev Anti Infect Ther. 2012 Mar;10(3):359-68. doi: 10.1586/eri.11.175. Review.

PMID:
22397568
38.

Guidelines for liver transplantation for patients with non-alcoholic steatohepatitis.

Newsome PN, Allison ME, Andrews PA, Auzinger G, Day CP, Ferguson JW, Henriksen PA, Hubscher SG, Manley H, McKiernan PJ, Millson C, Mirza D, Neuberger JM, Oben J, Pollard S, Simpson KJ, Thorburn D, Tomlinson JW, Wyatt JS; British Transplant Society.

Gut. 2012 Apr;61(4):484-500. doi: 10.1136/gutjnl-2011-300886. Epub 2012 Jan 10. No abstract available.

PMID:
22234978
39.

SLPI and inflammatory lung disease in females.

McKiernan PJ, McElvaney NG, Greene CM.

Biochem Soc Trans. 2011 Oct;39(5):1421-6. doi: 10.1042/BST0391421. Review.

PMID:
21936826
40.

ABO-incompatible pediatric liver transplantation in very small recipients: Birmingham's experience.

Gelas T, McKiernan PJ, Kelly DA, Mayer DA, Mirza DF, Sharif K.

Pediatr Transplant. 2011 Nov;15(7):706-11. doi: 10.1111/j.1399-3046.2011.01541.x. Epub 2011 Jul 15.

PMID:
21762327
41.

Long-term follow-up of children with 6-thioguanine-related chronic hepatoxicity following treatment for acute lymphoblastic leukaemia.

Rawat D, Gillett PM, Devadason D, Wilson DC, McKiernan PJ.

J Pediatr Gastroenterol Nutr. 2011 Nov;53(5):478-9. doi: 10.1097/MPG.0b013e31822960e9.

PMID:
21701407
42.

MR spectroscopy-based brain metabolite profiling in propionic acidaemia: metabolic changes in the basal ganglia during acute decompensation and effect of liver transplantation.

Davison JE, Davies NP, Wilson M, Sun Y, Chakrapani A, McKiernan PJ, Walter JH, Gissen P, Peet AC.

Orphanet J Rare Dis. 2011 May 9;6:19. doi: 10.1186/1750-1172-6-19.

43.

Stomal varices manifestation of portal hypertension in advanced intestinal failure-associated liver disease.

Iyer VB, McKiernan PJ, Foster K, Gupte GL.

J Pediatr Gastroenterol Nutr. 2011 May;52(5):630-1. doi: 10.1097/MPG.0b013e3182042917. No abstract available.

PMID:
21502833
44.

Primary prophylaxis of variceal hemorrhage in children with portal hypertension: a framework for future research.

Ling SC, Walters T, McKiernan PJ, Schwarz KB, Garcia-Tsao G, Shneider BL.

J Pediatr Gastroenterol Nutr. 2011 Mar;52(3):254-61. doi: 10.1097/MPG.0b013e318205993a. Review.

45.

Long-term care following paediatric liver transplantation.

McKiernan PJ.

Arch Dis Child Educ Pract Ed. 2011 Jun;96(3):82-6. doi: 10.1136/adc.2008.150656. Epub 2010 Oct 13. No abstract available.

PMID:
20943802
46.

Successful isolated liver transplantation in a child with atypical hemolytic uremic syndrome and a mutation in complement factor H.

Haller W, Milford DV, Goodship TH, Sharif K, Mirza DF, McKiernan PJ.

Am J Transplant. 2010 Sep;10(9):2142-7. doi: 10.1111/j.1600-6143.2010.03228.x. Epub 2010 Aug 11.

47.

Pre-emptive liver transplantation for primary hyperoxaluria (PH-I) arrests long-term renal function deterioration.

Perera MT, Sharif K, Lloyd C, Foster K, Hulton SA, Mirza DF, McKiernan PJ.

Nephrol Dial Transplant. 2011 Jan;26(1):354-9. doi: 10.1093/ndt/gfq353. Epub 2010 Jun 23.

PMID:
20573805
48.

Childhood autoimmune liver disease: indications and outcome of liver transplantation.

Chai PF, Lee WS, Brown RM, McPartland JL, Foster K, McKiernan PJ, Kelly DA.

J Pediatr Gastroenterol Nutr. 2010 Mar;50(3):295-302. doi: 10.1097/MPG.0b013e3181bf0ef7.

PMID:
20118802
49.

Improved outcomes of combined liver and kidney transplants in small children (<15 kg).

Perera MT, Silva MA, Sharif K, McKiernan PJ, Kelly DA, Lloyd C, Milford DV, Mayer DA, Mirza DF.

Transplantation. 2009 Sep 15;88(5):711-5. doi: 10.1097/TP.0b013e3181b29f0c.

PMID:
19741470
50.

Influence of ABCB1 polymorphisms and haplotypes on tacrolimus nephrotoxicity and dosage requirements in children with liver transplant.

Hawwa AF, McKiernan PJ, Shields M, Millership JS, Collier PS, McElnay JC.

Br J Clin Pharmacol. 2009 Sep;68(3):413-21. doi: 10.1111/j.1365-2125.2009.03461.x.

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